The Department of Health is today issuing the latest information about the numbers of known cases of Creutzfeldt Jakob disease. This includes cases of variant Creutzfeldt Jakob disease (vCJD) - the form of the disease thought to be linked to BSE. The position is as follows:

Referrals: a simple count of all the cases which have been referred to the National CJD Surveillance Unit for further investigation in the year in question. CJD may be no more than suspected; about half the cases referred in the past have turned out not to be CJD. Cases are notified to the Unit from a variety of sources including neurologists, neuropathologists, neurophysiologists, general physicians, psychiatrists, electroencephalogram (EEG) departments etc. As a safety net, death certificates coded under the specific rubrics 046.1 and 331.9 in the 9th ICD Revisions are obtained from the Office for National Statistics in England and Wales, the General Register Office for Scotland and the General Register Office for Northern Ireland.

Deaths: All columns show the number of deaths that have occurred in definite and probable cases of all types of CJD and GSS in the year shown. The figures include both cases referred to the Unit for investigation while the patient was still alive and those where CJD was only discovered post mortem (including a few cases picked up by the Unit from death certificates). There is therefore no read across from these columns to the referrals column. The figures will be subject to retrospective adjustment as diagnoses are confirmed.

Definite cases: this refers to the diagnostic status of cases. In definite cases the diagnosis will have been pathologically confirmed, in most cases by post mortem examination of brain tissue (rarely it may be possible to establish a definite diagnosis by brain biopsy while the patient is still alive).

Probable vCJD cases: are those who fulfil the 'probable' criteria set out in the Annex and are either still alive, or have died and await post mortem pathological confirmation. Those still alive will always be shown within the current year's figures.

Sporadic: Classic CJD cases with typical EEG and brain pathology. Sporadic cases appear to occur spontaneously with no identifiable cause and account for 85% of all cases.

Probable sporadic: Cases with a history of rapidly progressive dementia, typical EEG and at least two of the following clinical features; myoclonus, visual or cerebellar signs, pyramidal/extrapyramidalsigns or akinetic mutism.

Iatrogenic: where infection with classic CJD has occurred accidentally as the result of a medical procedure. All UK cases have resulted from treatment with human derived pituitary growth hormones or from grafts using dura mater (a membrane lining the skull). Familial: cases occurring in families associated with mutations in the PrP gene (10 - 15% of cases).

GSS: Gerstmann-Straussler-Scheinker syndrome - an exceedingly rare inherited autosomal dominant disease, typified by chronic progressive ataxia and terminal dementia. The clinical duration is from 2 to 10 years, much longer than for CJD.

vCJD: Variant CJD, the hitherto unrecognised variant of CJD discovered by the National CJD Surveillance Unit and reported in The Lancet on 6 April 1996. This is characterised clinically by a progressive neuropsychiatric disorder leading to ataxia, dementia and myoclonus (or chorea) without the typical EEG appearance of CJD. Neuropathology shows marked spongiform change and extensive florid plaques throughout the brain. Definite vCJD cases still alive: These will be cases where the diagnosis has been pathologically confirmed (by brain biopsy).

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7December 2006 are now available.

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64. A member noted that at the recent Neuroprion meeting, a study waspresented showing that in transgenic mice BSE passaged in sheep may be morevirulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiformencephalopathy (BASE) MAY BE RELATED. A mutation had been identified in theprion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO AMUTATION FOUND IN CASES OF SPORADIC CJD.

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE straindiscovered recently in Italy, and similar or different atypical BSE caseswere also reported in other countries. The infectivity and phenotypes ofthese atypical BSE strains in humans are unknown. In collaboration withPierluigi Gambetti, as well as Maria Caramelli and her co-workers, we haveinoculated transgenic mice expressing human prion protein with brainhomogenates from BASE or BSE infected cattle. Our data shows that about halfof the BASE-inoculated mice became infected with an average incubation timeof about 19 months; in contrast, none of the BSE-inoculated mice appear tobe infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent thanclassical BSE in humans.***

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost mymom to hvCJD (Heidenhain variant CJD)and have been searching for answers ever since. What I have found is that wehave not been told the truth. CWDin deer and elk is a small portion of a much bigger problem.”............................